Tumefactive demyelinating lesions: A Clinicopathological correlative study.

Tumefactive demyelinating (TDL) lesions are focal zones of demyelination in the central nervous system and they often mimic the neuroimaging features of an intraxial neoplasm. In this report we describe the clinical, neuroimaging and neuropathological features of six cases of TDL. Only in two patients the neuroimaging features in MRI (magnetic resonance imaging) scans were suggestive of TDL while in the other four cases a diagnosis of glioma was suggested. In order to establish a confirmatory diagnosis neuronavigation/stereotactic biopsy was undertaken and the diagnosis of TDL was established in all six cases at histopathology. Two out of six patients did not respond to the conventional corticosteroid therapy and they were treated with plasma exchange. It is being concluded that neuronavigation biopsy, though provide only a small amount of tissue, and is extremely useful in making the diagnosis of TDL.

Carotid artery stenting: results and long-term follow-up.

BACKGROUND AND PURPOSE: The role of carotid artery stenting (CAS) as an alternative to carotid endarterectomy in the treatment of for symptomatic carotid artery stenosis is investigated. MATERIALS AND METHODS: Forty-seven patients underwent CAS over 10-year period. Forty-nine vessels were treated. Stenosis quantification was done using North American symptomatic carotid endarterectomy trial method. The mean follow-up period by clinical and Duplex examination ranged is 5.6 years. RESULTS: The technical success rate was 100%. There were four deaths (8.1%) and two (4.1%) minor strokes within thirty days of procedure. There was no major strokes. All patients with minor stroke achieved complete recovery at 1-month follow up. Two deaths occurred probably due to hyperperfusion syndrome (HS) and two due to cardiac arrest. CONCLUSION: CAS is an effective treatment modality of symptomatic carotid artery disease but should be carefully done in high-risk groups having severe medical ailments and those having severe bilateral stenosis of the carotid arteries.

Satoyoshi syndrome.

Satoyoshi syndrome (Komuragaeri disease) is a rare disorder of presumed autoimmune etiology, characterized by painful muscle spasms, alopecia, diarrhea, endocrinopathy with amenorrhoea and secondary skeletal abnormalities. Most of the previous reports are of the Japanese people. We report the first case from India.

Intravenous immunoglobulin reduces serum tumor necrosis factor alpha in patients with Guillain-Barre syndrome.

BACKGROUND: Tumor necrosis factor a TNF-alpha has a possible role in the pathogenesis of the Guillain-Barre syndrome (GBS). AIMS: To study the effect of intravenous immunoglobulin (IVIg) on serum TNF-alpha concentrations in patients with GBS. MATERIAL AND METHODS: The effect of IVIg on TNF-alpha was evaluated in 36 patients with GBS. Serum TNF-alpha concentration was measured by enzyme-linked immunosorbent assay (ELISA). The sera of 22 (61%) patients with GBS showed elevated concentrations of TNF-alpha (35-182 pg/ml) and these sera were individually incubated in vitro with IVIg (0.25 mg/ml) at 37 degrees C for 24 hours. RESULTS: The serum TNF-alpha concentrations in the 22 GBS patients with elevated levels showed a steady decline (60.34-19.78 pg/ml) following incubation with IVIg. These 22 patients also received IVIg therapy, and serum TNF-alpha concentrations showed a significant decline (65.5-9.75 pg/ml) at the end of the therapy. At the time of discharge from the hospital, there was a positive correlation between neurological recovery and decline in TNF-alpha concentrations in these 22 GBS patients. CONCLUSIONS: The results of this study indicate that elevated levels of TNF-alpha occur in a proportion of patients with GBS and in these patients elevated serum TNF-alpha levels decline with IVIg therapy.

Circulating tumour necrosis factor alpha & soluble TNF receptors in patients with Guillain-Barre syndrome.

BACKGROUND & OBJECTIVES: Tumour necrosis factor-alpha (TNF-alpha) is regarded as one of the immune factors that can induce demyelination of peripheral nerves in patients with Guillian-Barre syndrome (GBS). This present study was undertaken to find out the role of TNF-alpha and soluble TNF receptors in the pathogenesis of GBS; and to study the effect of intravenous immunoglobulin (ivIg) therapy on the serum TNF-alpha and soluble TNF receptors in patients with GBS. METHODS: Thirty six patients with GBS in progressive stages of motor weakness were included in this study. The serum TNF-alpha and soluble TNF receptors (TNF-RI, TNF-RII) were measured in the serum samples of these patients before and after ivIg therapy by a sandwich ELISA. RESULTS: Of the 36 patients with GBS, 26 (72.2%) showed elevated serum TNF-alpha levels prior to ivIg therapy. Following a complete course of ivIg therapy there was a progressive decrease in the serum TNF-alpha concentrations in these 26 patients. On the other hand, the soluble TNF receptors, particularly TNF-RII showed an increase in the serum of GBS patients following ivIg therapy. INTERPRETATION & CONCLUSION: The results indicate that ivIg reduces the serum TNF-alpha concentrations in the GBS patients having elevated levels prior to ivIg therapy. Elevated serum levels of soluble TNF receptors following ivIg therapy may play a protective role by inhibiting the demyelinating effect of TNF-alpha in the peripheral nerves of patients with GBS.

Significance of serum antibody to GD1b ganglioside in patients with Guillain-Barré syndrome.

BACKGROUND & OBJECTIVES: The precise etiological factors in Guillain-Barré syndrome (GBS) are still unknown. However, humoral and cellular immune factors may have a role in the pathogenesis of GBS. The present study was undertaken to evaluate the clinical significance of circulating serum IgG antibody to GD1b ganglioside in patients with GBS. METHODS: Serial samples of serum were collected from 18 patients with GBS undergoing plasma exchange (PE) during their hospital stay. Serum IgG antibody titers to GD1b, before, during as well as following PE were measured by an indirect enzyme-linked immunosorbent assay (ELISA). RESULTS: In 10 of 18 patients with GBS the antibody to GD1b was present in high titers (1:640-1:5120) prior to PE and the antibody titers in these 10 patients decreased following PE. At the time of completion of the study, the anti GD1b antibody titers declined in relation to clinical recovery in 7 of 10 patients with GBS. INTERPRETATION & CONCLUSION: The findings of the present study show that antibody to GD1b gangliosides may be one of the immunological factors in the pathogenesis of GBS and PE decreases the anti GD1b antibody titers in these patients.

Mitochondrial myopathies--a clinicopathological study.

Mitochondrial myopathies are heterogeneous group of clinical disorders that can affect multiple systems besides skeletal muscles. The mitochondrial abnormalities in the skeletal muscles are morphologically identified by the presence of characteristic Ragged-red fibers (RRF) in the cryostat sections of the muscle stained with modified Gomori's trichrome stain. In this retrospective study, clinical and histopathological features in six patients with mitochondrial myopathies have been analysed. The utility of histochemical methods in confirming the diagnosis of mitochondrial myopathy has been emphasised.

Spinal muscular atrophy--a clinicopathologic analysis.

In this retrospective study the clinical features in 16 children with spinal muscular atrophy (SMA) were reviewed and classified into three stages. The muscle biopsy specimen were routinely processed with liquid-nitrogen-isopentane and 8 micron thick frozen-sections were studied for histochemical changes. The clinical features in Type III SMA resembled with limb-girdle muscular dystrophy and the muscle biopsy was useful in distinguishing these two entities. It is being evaluated that prenatal diagnosis of SMA is possible with DNA technology developed recently in our country.

Inflammatory myopathies--a clinicopathologic study.

In this study, clinical, histopathological and immunological profiles were analysed in ten patients with inflammatory myopathies. Polymyositis and dermatomyositis were more common than other forms of inflammatory myopathies. The pathogenetic mechanisms and distinguishing histopathological and immunological profiles between polymyositis and dermatomyositis have been highlighted.

Nitroimidazoles, Part XXIII--activity of satranidazole series against anaerobic infections.

A large number of nitroimidazoles have been examined for in vitro activity against three anaerobes - Bacteroides fragilis (Bf), a strain of Bf resistant to metronidazole (16a) and Clostridium perfringens and many found to be active. Among these may be mentioned 1-methyl-5-nitroimidazoles carrying N - bound hetetocycles at position 2, such as satranidazole 1a, 1b, 1c, 1k, 1n and 1v which are at least twice as active as metronidazole (16a), ornidazole (16b) and tinidazole (16c). Even more active are 5-nitroimidazolyl benzimidazole 5d, -thiazolidinone 6b and thiadiazolidine dioxide 8a. Many other types of compounds derived from 1-methyl-2-amino-5-nitroimidazole are feebly active. Among 5-nitroimidazoles with a carbon substituent at position 2, 16a, 16b and 16c are equiactive while dimetridazole 14f is more active than 16a against Bf. Some 2-vinyl derivatives are very potent, with 18f and 18i being outstanding. Activity better than that of metronidazole is seen for nitroimidazooxazepines, e.g. 29d. 5-Nitroimidazoles are more active against anaerobes than 4-nitro isomers. Antianaerobic and antiamoebic activities generally run parallel in these classes of compounds. The study has led to the elaboration of the antianaerobic profile of satranidazole 1a.